Yesterday was results day.. a mixed bag

So I went back to Papworth yesterday to get my results of recent tests, have been left with mixed feelings really.

CT scan showed no worsening since previous scan - air trapping and atelectasis with the obvious lung reduction from scoliosis but no evidence of bronchiectasis thankfully

LFT's showed the usual 11% functioning of small airways with lung volume of around 25%, despite this, gas exchange was as good as can be expected So, no apparent worsening.

Nasal nitric oxide was just in the normal range (8ppm over threshold) so that should safely rule out ciliary motility disorder which was being considered.

Heart echo showed right ventricular hypertrophy this wasnt present on my last echo about four years ago, and is due to the severity of my obstructive and restrictive lung disease. Trying to not think about that one too much. Having a nurse and doctor in the house isn't fun at times.

Bone density scan showed a reduction of 4% since the last one a couple of years ago (2 yrs is the minimum time between repeats). Really p'd off about this but its also unsurprising given my need for steroids. Will be starting IV infusions of bisphosphonate and have been given the usual advice about not falling over or doing silly things. Pants.

It seems then that a lot of my deterioration has been due to recurrent aspiration pneumonia and reflux - and consequent unstable asthma. This has improved a bit since being nil by mouth and getting an electric bed so I sit up at night, but waiting for an opinion now re jejunostomy so nothing goes in my stomach.

Im now dreading the summer because it usually means several months on oral steroids, and now I dont want to take the damn things because of my bones. Because of my spinal fusion and instrumentation osteoporosis is a biggy I wanted to avoid - I was always told that if I got it, it would cause big problems.

Also now being referred to the RSSC for a sleep study to see if I need night time ventilation (CPAP/BiPAP). If I need that, my life will be bloody complete. argh. Been referred because Nick has had to wake me because I've stopped breathing, and Im tired all the time no matter how much sleep I get. Im also high risk due to my scoliosis and lung volumes.

All in all a very mixed bag of a day, but at least some results were unchanged. Hardly normal, more consistently crap!

Need to do some weight bearing exercise now then

Lynda

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I just want to send massive hugs Lynda, your brain must be buzzing with all of that information!

Take care xx

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Add mine to that - thinking of you.

I'm crossing my fingers for you re the osteoporosis and sleep apnoea as you already have more than enough to deal with without adding those. Hoping you get some GOOD (as in more than 'no change') news soon as I think you deserve it!

xx

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Wow, that's a lot of stuff to be mulling over. I'm glad to hear that some things are not getting worse, but really feeling for you re the deterioration of heart, sleep and bones. Lots of hugs for you xx

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Sending my hugs too! WHat a lot of info to take in! My brain just starts to melt after a few key facts, id need about 7 appointments to get all that! good for you for remembering everything

Hopefully as Papworth have been so helpful for you they will have some good suggestions for how to help with bones/sleep/heart type issues. And hopefully these solutions will be fairly 'easy' ones to do

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Yeah it's been a bit to chew over. I'm hoping the IV infusions will slow the progression of the osteoporosis, they said they will re scan me sooner to check whether its having an effect. I just didn't expect it to have deteriorated

The heart thing - well it's best to stick head in large bucket of sand I find! If it changes over time it will only go one way. Nothing to do about it as I can't change my pulmonary function/issues.

That's okay, Im good at buckets of sand

Sleep study will be interesting, but cpaps are horrid and I don't want one for my hubby's sake as much as mine. We shall see... I'm waking up exhausted no matter how much sleep I get - have done for a long time, and Hubby has had to shake me to start me breathing again

Soph you made me chuckle lol. I'm used to Papworth appointments now but found them odd to start with! They are very long - sometimes up to an hour with consultant and other folk in the MDT see you too. I usually take a list and quiz them! It's a day trip...

The rest of me works okay - and Im still thankful there are no bronchiectatic changes, that's something to be happy about

Thanks it's good to be able to spill on here. I don't really say anything to friends/family.

Lynda

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Hi Lynda,

I've been an inpatient at Papworth for the past three weeks, so just wondering whether we have passed in the corridor?!

Glad to hear that your CT scan was unchanged. When I arrived at Papworth in September, having transferred my care, they initially 'undiagnosed' me with bronchiectasis as they used an old scan where most of the lung was invisible because of a large pneumothorax. Sadly a repeat scan in December confirmed that it is indeed present, and quite advanced, with typical 'ground glass' and 'tree in bud' changes (very poetic!). This is no surprise at all, as repeated bacterial infections and vast amounts of thick sputum are my major problem, so bronchiectasis is the obvious explanation.

As I think we've discussed before, my nasal nitric oxide was way off the normal range, at something like 25% normal levels. I went over to Glenfields for further tests, and although nobody has received official confirmation, the consultant at the time did confirm that his brief check under the microscope suggested all was normal. Such low levels normally indicate PCD or CF, but I guess in my case they probably indicate nothing at all!

Sorry to hear about the reduction in bone density. Was the previous one also done at Papworth, because it could perhaps be suggestive of a different machine as much as anything. 4% over two years should give you some time (depending on your starting level of course!) and hopefully the new infusions will turn things round for you.

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Hiya Carrie, which ward do you use? At the moment, if Im in, I go on Princess, they are so lovely on there. Three weeks is a bummer, any idea when they are letting you out? Really sorry you are dealing with bronchiectasis, its a horrid thing to have. Thankfully I have no trees in bud right now.. (what a ridiculous expression, it makes it sound like something nice!!). Hopefully the consultant at Glenfields didn't just do the preliminary look under the microscope, but sent the samples off for analysis too...

Both my bone dexa's have been done at Papworth, so unfortunately I cant blame the difference on different machines Im so hoping the infusions will slow its progression. My lungs are already worse because of the change in weather (and pollen) so Im dreading the coming months as it meant six months of oral steroids last year - not what my bones need! We took the dog out earlier and I was really struggling, even after a 5mg neb.

I dont know many on here who go to Papworth, so will have to give you a heads up next time Im there, to see if you are too! I do hope you get out soon though

Lynda

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Posting in two halves as I keep deleting my posts - aaaggggghhh!

When I was admitted to Papworth three weeks ago, I was in the middle of a serious infective asthma exacerbation, and I was started on continuous bipap as I was unable to maintain my oxygen saturations any other way. I have to say - I totally love it! Obviously the fact that it enabled me to avoid intubation might have made me somewhat biased, but I do find it so helpful - I use it when I'm tired or struggling, to help catch my breath when wheezing and tight, and for physiotherapy and to support my lungs when I'm trying to exercise other muscles. And at night, of course. Like you, I'm not that keen on the idea of it in the marital bedroom, but I suppose so be it.... I don't find it difficult to sleep with at all, although do make sure you try out a good range of masks, as the first one they gave me left me seeing double!

Your lung function is ever so low. I am enormously impressed with how well you function like that. Is it quite stable, and would transplant ever be an option for you? Like you, I would just bury my head in the sand about the heart thing. Papworth would surely refer you to their cardiac/pulmonary hypertension colleagues if/when treatment would be helpful, and until then, there is not a lot you can really do about it so best not to lose sleep over it! Easier said than done though, and it doesn't sound as though you have a lot of sleep to lose!

Good luck - and keep well.

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Posting in two halves as I keep deleting my posts - aaaggggghhh!

LOL

Thanks so much for your second post, its really reassuring to hear that someone actually feels positive about their ventilation! So, from the sound of your post, you dont just use it at night then, but for helping you with other things? I didn't realise that people did that, its really interesting. So, are you on RSSC at the moment then, or do they just do the assessment and setting you up? Thanks for the heads up about the masks, I will bear that in mind if I do end up needing it. My hubby is fairly philosophical about it being in the bedroom, its me that hates the thought of it. But I guess so would he if the boot was on the other foot.. we shall deal with it if it comes, just like everything else I guess. Im amazed you dont find it difficult to sleep with - again thats very reassuring.

Yeah, my lung function is diabolical lol. Papworth are also amazed at how well I cope given how limited it is. I do wonder if its because its been lifelong, rather than a sudden deterioration, so they have learned to compensate? I dont know. The biggest issue is that because I have no reserves, things go wrong very fast at the slightest thing - pollen, infection, asthma etc. An asthma attack with a litre of lung function isnt that much fun! Transplant isn't an option because my thoracic space is so small and distorted by my kyphoscoliosis, that it wouldnt fit in there - and if it did, it would still be restricted by my shape. Hope that makes sense, I know what I mean..

I have no doubt that Papworth would refer me back to Cardiology if need be. I was seen by cardiology a couple of years ago for SVT - they did the ablation procedure but it was unsuccessful - it was too widespread due to my lung disease. Its amazing just how much lung issues can affect your heart. Like you say, bucket of sand, and dont lose sleep over it!

I hope you're starting to feel better and you get home soon. Thanks so much for your post

Lynda

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I'm still on Princess ward, but then I am still acutely unwell, starting my third set of IV antibiotics for my chest. Because I am so unwell and unable to maintain my oxygen saturations on just mask/nasal specs alone, I am using the NIV almost continuously at the moment, taking it off just to eat. I am hoping that this will improve as I and my lungs get stronger.

Hope things get sorted for you soon.

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Oh dear you are in a pickle. I'm in outpatients wed and Thurs this week, for oesophageal Manometry and 24hr ph monitoring. Be good to come and say hi but I tend to avoid folks with infections at all costs! I'm sure you can understand that. Blimey I do hope you're feeling better soon, at least you're in a decent place - I saw today that Papworth scored about 20% higher than the national average for patient satisfaction, both doctors and nurses scored 9.something out of ten

Get well soon

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Absolutely would want you to avoid me and my 'rare and opportunistic pathogenic bacteria'! These antibiotics are definitely helping though, as both blood tests and my general well being is demonstrating, so a massive relief at last!

Remind me, do you have a jejeunostomy? That is next on their plans. NG Feeding has Enabled me to get my BMI up, but all bacteria since starting have been gut bugs suggesting I could well be aspirating. I think you said this was your problem? Will start hitting google tomorrow to find out more.

Hope tomorrow and Thursday goes well.

Xx

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Absolutely would want you to avoid me and my 'rare and opportunistic pathogenic bacteria'! These antibiotics are definitely helping though, as both blood tests and my general well being is demonstrating, so a massive relief at last!

Remind me, do you have a jejeunostomy? That is next on their plans. NG Feeding has Enabled me to get my BMI up, but all bacteria since starting have been gut bugs suggesting I could well be aspirating. I think you said this was your problem? Will start hitting google tomorrow to find out more.

Hope tomorrow and Thursday goes well.

Xx

Great news about the ABX working

I started with a PEG (thank god I never had to do NG feeding, I have a tube up my nose at the moment for the 24hr testing and its driving me NUTS) and got my BMI up from 14 to 18 but issues with constant aspiration pneumonia have been a real problem, even tho I sleep almost upright with an electric profiling bed. So, I've just been back to Addenbrookes - was re referred by Dr Barker, and the decision has been made to give me a jejunal extension (I need to keep the stomach part for venting my stomach) so that nothing at all is going in my stomach then feed wise. Hopefully that will stop the aspiration pneumonia! With hindsight I could have had a a Jej placed rather than a PEG initially but the PEG has additional benefits for me, and I guess who was to know that my reflux would get so bad.

In theory, its a great plan, Im hoping in practice it is too... Sounds like a very sensible suggestion on their part for you, if they think it is reflux/aspiration related. I stopped feeding at night because it was just too risky, but I cant tolerate a high enough feed rate to have all my feed in the day, so Im losing weight again. Hence Im inpatient for it to get sorted.

Glad you're starting to feel a bit better xx

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Sorry to hear about the bone density, heart issues and the need for the sleep study.

I wish things were different for you.

Hoping you get the best care possible so that these things don't interfere with your life (or bedroom) any more than absolutely necessary.

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Really sorry to read you are still in hospital.

Fingers crossed that its third time lucky for the IV's.

Get well soon x